Key Points
Î Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive,
fibrosing interstitial pneumonia of unknown cause occurring in adults.
Î Radiologic and/or histopathologic patterns are consistent with usual
interstitial pneumonia.
Î Significant advances have been recently made in the clinical management
of IPF.
Î All of the recommendations in favor of treatment are conditional, meaning
that decisions to treat patients with IPF should be individualized. Clinicians
should be cautious to not interpret recommendations with the same grade as
having the same net effects.
Î The recommendations in favor of treatment put a higher value on the
potential benefit of patient-important outcomes such as disease progression
as measured by FVC decline and mortality and a lower value on potentially
significant adverse effects and the cost of treatment.
Treatment
Table 1. Recommended Drugs
a
Brand Generic How Supplied Dose
Esbriet
®
Pirfenidone 267 mg capsules With food:
Day 1-7: 1 capsule tid
Day 8-14: 2 capsules tid
Day 15 onward: 3 capsules tid
OFEV
®
Nintedanib 150 mg capsules
100 mg capsules
With food:
150 mg capsule q12h
(Temporary interruption or
reduction to 100 mg bid for
adverse effects)
a
Most of the evidence that provided the foundation for the suggestion to use regular antiacid
therapy was derived from patients receiving proton pump inhibitor therapy. A very small
proportion received histamine-2 receptor antagonists. Other antiacid treatments may need to
be considered differently.
